ReviewClinical assessment of Tourette syndrome and tic disorders
Highlights
► Proper Tourette syndrome assessment must include evaluation of common comorbidities. ► Most patients with Tourette syndrome experience premonitory urges prior to tics. ► Rating scales exist to measure severity of tics and associated sensory phenomena. ► Most children with Tourette syndrome improve during adolescence and early adulthood.
Introduction
Tourette syndrome (TS) was first described by the French neurologist, Gilles de la Tourette, in 1885 as a “maladie des tics.” In his original case series describing the syndrome that now bears his name, Gilles de la Tourette wrote about many of the characteristics of the syndrome including: involuntary movements and sounds, markedly enhanced startle reactions, a tendency to repeat both vocalizations (echolalia) and movements (echopraxia), and uncontrollable verbal obscenities (coprolalia) (Lajonchere et al., 1996). Since then, our knowledge of TS has progressed significantly, including advances in our understanding of tics, their surrounding sensory phenomena, and the central role that other co-occurring diseases, such as attention deficit hyperactivity disorder (ADHD) and obsessive-compulsive disorder (OCD), have on the overall clinical course of the disorder. This review will focus on our current understanding of the diagnosis, clinical characterization and assessment of tics as well as their clinical course. Other reviews will focus on the evidence-based treatment and neurobiology of tic disorders.
Section snippets
Definition of tics
Tics appear as sudden, rapid, purposeless motor movements or sounds that involve discrete muscle groups. They are also stereotyped in that they will occur in a similar manner each time they are performed. In comparison to some movement disorders or psychiatric conditions (e.g. sterotypies, chorea, or dyskinesia), patients with tics report the ability to suppress them, even if only for a short duration. However, they report that suppression often causes discomfort. Almost any movement, sound, or
Tourette syndrome and other tic disorders
The prevalence of TS varies based on study design and location. An international prevalence of 0.6–1% has been reported for mainstream schoolchildren, with the disorder being 3–4 times more common in males than in females (Cavanna and Termine, 2012). Data from the 2007 National Survey of Children's Health (NSCH) showed an estimated prevalence of 0.3% among U.S. children aged 6–17 years (Scahill et al., 2009). This number may represent an underestimate of TS prevalence since data were gathered
Characterization of tics
Tics are characterized by their anatomical location, number, frequency, and duration. They are also further described by their forcefulness or intensity and by their complexity (ranging from simple to complex). The most widely-used rating scale of tic severity is the Yale Global Tic Severity Scale (YGTSS), which includes separate scores from 0 to 5 for number, frequency, intensity, complexity, and interference (the degree to which planned actions or speech are interrupted by tics) of both motor
Natural history
The natural history of TS has been established based on clinical observations. There is a clear progression of the disorder from the onset of symptoms to, in most cases, full or partial regression of symptoms. Tics usually begin around 6–8 years of age, and 90–95% of TS cases have an onset of tics between the ages of 4 and 13 (Leckman et al., 1998). Simple motor tics involving the eyes or face are usually the first to appear in a child with TS. They are called simple because they involve a
Sensory phenomena surrounding tics
The outward manifestation of TS represents only a part of the symptomatology experienced by most of our patients. In 1980, Joseph Bliss, articulately described his careful observations from 35 years of self-study of the feelings and subjective events surrounding his own tics. Much of what he described became the basis for future research surrounding the sensory phenomena associated with tics. The term, “sensory phenomena,” is now used as an all-encompassing term to describe such subjective
Exacerbating/alleviating factors
Tic symptoms vary in frequency and intensity, and in addition to potential neurological variation, it has been shown that certain environmental or contextual factors will either exacerbate or alleviate tic symptoms in individuals with TS.
The results of 6 different descriptive studies looking at the effects of different antecedent variables on tic severity show stress and anxiety appear to be the most common factors associated with an increase in TS symptoms, while fatigue and boredom also rank
Suppressing tics
One of the characteristics of tic symptoms is that they are suppressible, even if only for a short while. However, as stated earlier, the act of suppression can lead to the build-up of uncomfortable premonitory urges. In one study, 3 of 4 children who demonstrated reliable suppression showed a pattern of higher subjective urge ratings during suppression as compared to baseline (Himle et al., 2007).
Although tics can be suppressed, to do so requires more attention and energy from the individual.
Comorbidities
The description of behavioral and emotional disturbances in patients with TS has occurred since 1899, around the time the disorder was first described by Georges Gilles de la Tourette himself (Coffey and Park, 1997). In fact, comorbid neuropsychiatric disorders, the majority being ADHD and OCD, have been shown to occur in up to 90% of TS patients in both clinic and community settings (Wright et al., 2012). Figure 2 depicts the time course of common comorbidities in relation to tic symptoms, as
Conclusions
Tourette's syndrome is a neuropsychiatric disorder characterized by multiple motor and vocal tics. However, for many individuals with TS, the tics are neither the most prominent nor distressing part of the disorder. In the majority of children with TS, tic symptoms diminish significantly during adolescence. Most individuals with TS experience associated sensory phenomena such as premonitory urges and somatic hypersensitivity that are often as distressing as the tics themselves. The majority of
Conflict of interest
The authors have no conflicts of interest to disclose.
Acknowledgements
The authors acknowledge the National Institute of Mental Health support of the Trichotillomania Learning Center (MHB), the Yale Child Study Center Research Training Program (MHB), the National Institutes of Health (K23MH091240 (MHB), T32MH018268-26 (JFL) and R25 MH077823 (JFL)), the APIRE/Eli Lilly Psychiatric Research Fellowship (MHB), the AACAP/Eli Lilly Junior Investigator Award (MHB), NARSAD (MHB), and UL1 RR024139 from the National Center for Research Resources, a component of the National
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